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Blood, 1 June 2004, Vol. 103, No. 11, pp. 4023-4027. Prepublished online as a Blood First Edition Paper on February 5, 2004; DOI 10.1182/blood-2003-11-3758. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-11-3758v1 103/11/4023    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Quinn, C. T. Articles by Buchanan, G. R. Search for Related Content PubMed PubMed Citation Articles by Quinn, C. T. Articles by Buchanan, G. R. Related Collections Red Cells Free Research Articles Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Survival of children with sickle cell disease Charles T. Quinn, Zora R. Rogers, and George R. Buchanan From the Division of Hematology-Oncology, Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas, and Center for Cancer and Blood Disorders, Children's Medical Center Dallas, Dallas, TX. Contemporary survival data are not available for children with sickle cell disease (SCD). The few previous childhood SCD cohort studies do not reflect the benefits of modern therapy. We defined an inception cohort of newborns with sickle cell anemia (SS), sickle-°-thalassemia (S °), sickle-hemoglobin C disease (SC), or sickle-+-thalassemia (S+) who were identified by newborn screening and followed for up to 18 years. The incidence of death and stroke were calculated. Overall survival, SCD-related survival (considering only SCD-related deaths), and strokefree survival were determined. The 711 subjects provided 5648 patient-years of observation. Twenty-five subjects died; mean age at death was 5.6 years. Five patients died from infection. Thirty had at least one stroke. Among SS and S° subjects (n = 448), the overall rates of death and stroke were 0.59 and 0.85/100 patient-years. Survival analysis of SS and S° subjects predicted the cumulative overall, SCD-related, and stroke-free survival to be 85.6%, 93.6%, and 88.5% by 18 years of age. No SCD-related deaths or strokes occurred in SC or S+ subjects (n = 263). Childhood mortality from SCD is decreasing, the mean age at death is increasing, and a smaller proportion of deaths are from infection. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Returns on long-term investments in sickle cell disease Alan R. Cohen Blood 2004 103: 3996. [Full Text] [PDF] This article has been cited by other articles: A. Hryniewicz-Jankowska, P. K. Choudhary, L. P. Ammann, C. T. Quinn, and S. R. Goodman Monocyte Protein Signatures of Disease Severity in Sickle Cell Anemia Experimental Biology and Medicine, February 1, 2009; 234(2): 210 - 221. [Abstract] [Full Text] [PDF] M Howard-Jones, L Randall, B Bailey-Squire, J Clayton, and N Jackson An audit of immunisation status of sickle cell patients in Coventry, UK J. Clin. Pathol., January 1, 2009; 62(1): 42 - 45. [Abstract] [Full Text] [PDF] C. T. Quinn, K. Thomas, Z. R. Rogers, and G. R. Buchanan Improved Survival of Children and Adolescents with Sickle Cell Disease. Blood (ASH Annual Meeting Abstracts), November 16, 2008; 112(11): 1425 - 1425. [Abstract] [Full Text] C. T. Quinn, N. J. Lee, E. P. Shull, N. Ahmad, Z. R. Rogers, and G. R. Buchanan Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort Blood, January 15, 2008; 111(2): 544 - 548. [Abstract] [Full Text] [PDF] M. Mazumdar, M. M. Heeney, C. M. Sox, and T. A. Lieu Preventing Stroke Among Children With Sickle Cell Anemia: An Analysis of Strategies That Involve Transcranial Doppler Testing and Chronic Transfusion Pediatrics, October 1, 2007; 120(4): e1107 - e1116. [Abstract] [Full Text] [PDF] F. Bernaudin, G. Socie, M. Kuentz, S. Chevret, M. Duval, Y. Bertrand, J.-P. Vannier, K. Yakouben, I. Thuret, P. Bordigoni, et al. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease Blood, October 1, 2007; 110(7): 2749 - 2756. [Abstract] [Full Text] [PDF] A. D. Villella The Effect of the Pneumococcal Conjugate Vaccine on Children with Sickle Cell Disease AAP Grand Rounds, July 1, 2007; 18(1): 1 - 2. [Full Text] [PDF] I. Roberts and M. de Montalembert Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in Europe Haematologica, July 1, 2007; 92(7): 865 - 871. [Full Text] [PDF] P. Telfer, P. Coen, S. Chakravorty, O. Wilkey, J. Evans, H. Newell, B. Smalling, R. Amos, A. Stephens, D. Rogers, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London Haematologica, July 1, 2007; 92(7): 905 - 912. [Abstract] [Full Text] [PDF] G. R. Buchanan and M. J. Kahn Hemolytic anemias ASH Self-Assessment Program, January 1, 2007; 2007(1): 102 - 142. [Full Text] [PDF] C. T. Quinn, E. P. Shull, N. Ahmad, N. J. Lee, Z. R. Rogers, and G. R. Buchanan Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia Blood, January 1, 2007; 109(1): 40 - 45. [Abstract] [Full Text] [PDF] C. T. Quinn, N. J. Lee, E. P. Shull, and G. R. Buchanan Prediction of Adverse Outcomes in Children with Sickle Cell Anemia: Testing the CSSCD Early Prediction Model in the Dallas Newborn Cohort. Blood (ASH Annual Meeting Abstracts), November 16, 2006; 108(11): 3796 - 3796. [Abstract] [Full Text] [PDF] L. A. Smith, S. O. Oyeku, C. Homer, and B. Zuckerman Sickle Cell Disease: A Question of Equity and Quality Pediatrics, May 1, 2006; 117(5): 1763 - 1770. [Abstract] [Full Text] [PDF] C. T. Quinn, N. J. Lee, Z. R. Rogers, and G. R. Buchanan Survival of Children with Sickle Cell Disease: An Update from the Dallas Newborn Cohort. Blood (ASH Annual Meeting Abstracts), November 16, 2005; 106(11): 3812 - 3812. [Abstract] J. R. Botkin Research for Newborn Screening: Developing a National Framework Pediatrics, October 1, 2005; 116(4): 862 - 871. [Abstract] [Full Text] [PDF] C. T. Quinn, Y.-P. Chin, N. Ahmad, Z. R. Rogers, and G. R. Buchanan Prognostic Significance of Birth Weight and Red Blood Cell Phenotype in Sickle Cell Disease. Blood (ASH Annual Meeting Abstracts), November 16, 2004; 104(11): 3756 - 3756. [Abstract] A. D. Villella Improved Survival for Children with Sickle Cell Disease AAP Grand Rounds, October 1, 2004; 12(4): 41 - 42. [Full Text] [PDF] G. R. Buchanan, M. R. DeBaun, C. T. Quinn, and M. H. Steinberg Sickle Cell Disease Hematology, January 1, 2004; 2004(1): 35 - 47. [Abstract] [Full Text] [PDF] J. E. Sadler, J. L. Moake, T. Miyata, and J. N. George Recent Advances in Thrombotic Thrombocytopenic Purpura Hematology, January 1, 2004; 2004(1): 407 - 423. [Abstract] [Full Text] [PDF]

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